Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) protein, an epithelial chloride channel expressed in the airways, pancreas, testis …

Although there has been impressive progress in the elucidation of the genetic and molecular
basis of cystic fibrosis (CF), the pathogenesis of CF lung disease remains obscure. The …

The cellular mechanisms by which loss-of-function mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) chloride channel produce cystic fibrosis (CF) …

Cystic fibrosis (CF) is a common, life-threatening, multisystemic, autosomal recessive
disorder. In the last few years, giant steps have been made with regard to the understanding …

Pilewski, Joseph M., and Raymond A. Frizzell. Role of CFTR in Airway Disease. Physiol.
Rev. 79, Suppl.: S215–S255, 1999.—Cystic fibrosis (CF) is caused by mutations in the gene …

Cystic fibrosis (CF) is caused by the loss of functional CFTR Cl− channels. However, it is not
understood how this defect disrupts salt and liquid movement in the airway or whether it …

Despite an increased understanding of the cellular and molecular biology of the CFTR Cl−
channel, it is not known how defective Cl− transport across airway epithelia causes chronic …

Cystic fibrosis (CF) is a life-limiting disease characterised by recurrent respiratory infections,
inflammation and lung damage. The volume and composition of the airway surface liquid …

CFTR is a cAMP-activated chloride and bicarbonate channel that is critical for lung
homeostasis. Decreases in CFTR expression have dire consequences in cystic fibrosis (CF) …

Cystic fibrosis (CF) lung disease involves chronic bacterial infection of retained airway
secretions (mucus). Recent data suggest that CF lung disease pathogenesis reflects the …